Mesenchymal Chondrosarcoma

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Mesenchymal Chondrosarcoma
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introduction

• Mesenchymal chondrosarcoma, first discirbed by
  Lichtenstein and Bernstein cancer 1959
  121142-1157
• Rare, poor prognosis and long-term follow-up is
  necessary
• Primitive multipotential primary sarcoma Hutter
  and associates(cancer 1966191-25)
• Polyhistoma proposed by jacobson(cancer 1977
  402116-2130)

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• Very rare form of chondrosarcoma
• ???? maxilla, Mn, rib
• ???? 10?? 30???
• Equal gender distribution
• Radiologic app lytic lesion, most contain
  stipledd or large areas of calcification
• Highly metastatic potential and long term
  prognosis is extremely poor

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Major Problems of MCS

• High Malignant potentiality, especilly a tendency
  of metastasis, either earlier or later
• Tumor recurrence after long duration
• Low survival rate

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Clinical finding

• Swelling or pain or both, compression of neural
  structure(eg. Paresthesia)
• Hypesthesia, paralysis, pain after hard bite,
  bleedin sore, bleeding, epitaxis, nasal
  obstruction, numbness, difficulty hearing,
  painless swelling, Mass
• Painless swelling(28.6),mass(23.8),nerve
  disturbance(23.8)
• - Takahashi et al. Head Neck
  199315459-464

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duration

• 4 day to 7years and more than 2 years in 16 case-
  Yasuaki Nakashima et al,cancer 57 2444-2453,
  1896
• 3 week to 10 years- Takahashi et al. Head Neck
  199315459-464
• 1 week to 6 years-Vencio et al , Americna cancer
  society 19982350-55

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????

• In jaw, 11Mx 8Mn -Vencio et al , Americna cancer
  society 19982350-55
• 22Mx20Mn Takahashi et al
• Rib and jaw are most common site-Christensen Re
  1995

Nakashima et al
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Roentgenographic Findings

• lytic and destructive appearances with mottled
  calcification
• Sclerosis
• Cortical thickening
• Flocculent or stippled calcific densities ( soft
  tissue)
• On MRI, the mallignancy showed signal intensity
  lower than or equal to the brain On T1-weight
  image, and it became more isointense on
  T2-weighted image, with moderate heterogenous
  enhancement of the noncalcified
  component-Yasunori et al. JOMS 1999 57733-737

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Sexual

• 52(M)52(F)-no significant sex perdilection-
  Yasuaki Nakashima et al,cancer 57 2444-2453,
  1896
• 2418, ratio of 43-Takahashi et al. Head Neck
  199315459-464
• 109 -Vencio et al , Americna cancer society
  19982350-55

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Age of patient

• 5-71, approximately 60 of them were in the
  second and third dedades of life Yasuaki
  Nakashima et al,cancer 57 2444-2453, 1896
• 8 to 70, mean age(27), 71.4? 20?? 30?
• - Takahashi et al. Head Neck 199315459-464
• 2-51years, 16 Pt lt 30 years(mean 19)
• - Vencio et al ,. American cancer society
  19982350-55

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????

• Bone scan
• CT, MRI
• Incisional Biopsy
• A fine needle aspiration biopsy
• immunohistochemistry?the undifferentiated stromal
  cell of MCS typically express CD99 and vimentin,
  whereas S100 is usually more focally expressed in
  the areas of cartilagenous differentiation

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Pathologic findings

• Gloss finding
• Glossly, tumor were soft to firm and gray-white
  to reddish tan and frequently contained focal
  calcific deposits tumor varied from 3 to 22cm in
  greater diameter- Yasuaki Nakashima et al,cancer
  57 2444-2453, 1896

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Pathologic findings

• Microscopic finding
• the lesion were typically characterized by
  highly cellular proliferation of undifferentiated
  small cell, alternating with zone of
  differentiated cartilaginous tissue
• In cellular areas, undifferentiated cell with
  oval nuclei frequently tended to be arranged in a
  vague alveolar pattern of a herringbone pattern
• Large zone with solid sheets of small, round
  cell, similar to those seen in Ewings tumor,
  often were present

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Pathologic findings

• Microscopic finding
• Hemangiopericytoma-like arrangement characterized
  by clustering of cells around vascular spaces or
  by compartmentalization of closely packed cell
  with large clefts was another prominent
  histologic pattern observed in most tumor
• Chondroid matrix, osteoid, necrosis(inconspiculus)
  
• - Yasuaki Nakashima et al,cancer 57
  2444-2453, 1896

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treatment

• Intralesional excision(curettage for bone lesions
  and contaminatd excision for soft tissue
  tumor)-33 Pt, recurrence(80.5)
• Wide local excision-22 Pt, 40.9 reC
• Amputation-8 Pt, 70.5 reC
• Irradiation with or without chemotherapy-6 Pt
• Radiation therapy or chemotherapy or both after
  initial surgery-37 Pt

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prognosis

• In a group of 23 patient from the Mayo Clinic,
  the 5-year survival rate was 54.6 and the
  10-year survival rate was 27.3
• - Yasuaki Nakashima et
  al,cancer 57 2444-2453, 1896
• 5-year and 10-year survival rate of 42 and 28
• 
  -Huvos et al
• 58 survival late -Takahashi et
  al
• 5-,10-,15-year survival rate82,56,37.5

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Recurrence

• ?? bone ? lung ?? ??
• Breast, scalp, vulva, pelvis, lower back,
  cervical lymph node

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Soft tissue hard tissue

• 7238(boneextraskeletal), both and soft
  tissue1patient, 22 patient(craniofacial bone)
• -Yasuaki Nakashima et
  al,cancer 57 2444-2453, 1896

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discussion

• Ablative surgical treatment seemed to be the
  procedure of choice
• Several author suggested that recent
  chemotherapeutic regimens with or without
  radiation were efective in some cases of
  mesenchymal chondrosarcoma
• -Yasuaki
  Nakashima et al,cancer 57 2444-2453, 1896

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case report reviews
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Mesenchymal chondrosarcoma of the jaw

• 35/F(1983)
• 1month history of hypersthesia of the lower lip,
• Slight, firm swelling of the left Mn midbody
• Rx-osteolytic defect of midportion fo left
  horizontal ramus, 3.52cm, ground-glass
  appearance
• Wide local recection of Mn with Lt supra hyoid
  lymph node dissection chemotherapy
• 6 M later, T-12
• No F/up, 1988 died(multiple bone meta)
• Head neck
  199315459-464

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Mesenchymal chondrosarcoma of the orbit

• 45/M
• Dull aching pain in the right infraorbital
  region(about 2 months ago), protosis Rt(1year)
• A fine needle biopsy-small undifferentiated tumor
  cells
• Infraorbital biops? refered for radiotherapy and
  chemotherapy(no resection)
• -S.Kashyap et al, orbit
  2001,vol 20163-67

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Painful,rapidly growing mass of the mandible

• 18/F
• Painful swelling on the right side of Mn, facial
  asymmetry(about 3month), no lymph node
  enlargement, restricted jaw opening,no purulent
  drainage
• Rx large destructive, mixed radiolucent/radiopaqu
  e lesion
• Incisional biopsy?MCS
• Partial mandiblectomy,
• No F/up, 18??? ??
• -Haytham F, Oral surg Oral Med Oral
  Pathol Oral Radiol Endod 2003957-11

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Mesenchymal Chondrosarcoma of the Maxilla Report
of a case

• 13/f
• C/C Painless swelling of Rt. Maxilla( ? 2????
  ??)
• P/E facial asymmetry, no palpable cervical lymph
  node, elastic and soft well circumscribed
  mass(53cm), slight tenderness(), 12 13 MO(),
• Radio well circumscribed radiolucencyMRIlow
  signal intensity on T2-weight
• Tx chemotherapy partial Maxillectomy with wide
  surgical margin
• No evidence of recurrence or metastasis(8 month
  later)
• -Ariyoshi and shimahara Joms
  1999 57733-737

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table
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Mesenchymal chondrosarcoma of the maxilla

• 15/F
• C.C bilateral nasal obstruction, watery
  rhinorrhoea and slight epitaxis
• P.E No neck lymphadenopathy, large mass on hard
  palate(2/3)firm, no tender
• CT large mass in lower ant. Part of Rt.antrum.
  Peripheral calcification
• Tx right maxilletomy radiotherapy(60 Gy in
  7week), chemotherapy
• ?? 10?? ? fungal infection ?? ??
• -Bottrill et al , JLO
  1994 vol.108,pp.785-787

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Mesenchymal chondrosarcoma of the maxilla

• 14/F
• C.C right sideed nasal obstruction and snoring
  of 10 months duration
• CT.MRI
• Pre-OP chemotheray partial maxillectomy post-OP
  radiotherapy
• Disease free at 12month after surgical TX
• -Chidambaram et al, JLO
  2000,vol.114,pp. 536-539

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Case I

• ?OO

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• 24/F
• C.C
• induration on left cheek area find out 10 days
  ago
• mouth opening disturbance on left TMJ area

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• P.I
• lt. soft cheek infratemporal fossa mass
  about 43cm size, td(), slight movable
  consistent texture
• stensens duct saliva(), cervical LAP(_), but
  retromandubular TD(), numbness() on
  31,32,33,34 , lower lip, tongue(1/2) lt
  hypoesthesia
• 1years ago lt lower lip numbness10? ??? ??? ??
  ??? ?? ?? local ??????? lt tongue ½
  numbnessslight facial aymmetry due to lt facial
  swelling

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• F.H. ??? 2?? lung Ca? ??
• 93.11.26 waters, skull PA
  incisional Bx? accessory salivary gland
• 93. 12.15 tumor extirpation surgical excision
  with marginal resection from infratemporal fossa
  Lt.

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93.11.30
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93.12.10 B S
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1993.12.8 CT and 1993.12.3 MRI
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OP finding

1. Round to oval shape soft mass
2. Well-encapsulated on inferior medial portion
   but irregular margin on lateral superior
   portion
3. Calcifying material contained in tumor mass
4. Easily broken mass
5. About 654(depth) sizedlt T4(a) mass

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Post OP CT
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94.6.8
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• 1994.7.4 infraorbital pain, aspiration from sinus
  pus like exudate?CLOP

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95.8.14
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• 1995.11.14 Gap arthroplasty by condylectomy
  Lt.Coronoidectomy,Lttemporal diskopexy of
  miniscusLt. Max sinus O-A fistula closure with
  frozen biopsy of post wall massfrozzen biopsy
  internal mass of condyle ramus

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95.11.17
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96.2.1
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• 1996.2.13condylectomyO-A fistula
  closurefistulectomy with curettage of
  osteomyelitic lesion

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96.2.16
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96.5.11
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• 1996.5.23frozen biopsy of previous OP
  sitefistulectomy with coronoidectomycurettage
  of osteomyelitis

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96.5.30
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2000.9.19
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2001.4.20
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2003? 6? 3? CT
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referernces

• 1)Nakishima Y, Unni KK, shives TC, Swee RG,
  Dahlin DC.Mesinchymal Chondrosarcoma of bone and
  soft tissue. A review of 111 case.cancer
  1986572444-53
• 2)Takahashi mensencymal chondrosarcoma of the
  Jaw-report of a case and review of 41cases in the
  literature. Head Neck 199315459-464
• 3) Vencio et al , Mesenchymal Cnondrosarcoma of
  jaw bones. americal cancer society 19982350-55
• 4) S.Kashyap et al Mesenchymal Chondrosarcoma of
  the ortita clincopathological study.
  Orbit-2001,vol.20 No.I,pp.63-67
• 5) Haytham F, Oral surg Oral Med Oral Pathol Oral
  Radiol Endod 2003957-11
• 6) Ariyoshi and shimahara Joms 1999 57733-737
• 7) Bottrill et al , JLO 1994 vol.108,pp.785-787
• 8) Chidambaram et al, JLO 2000,vol.114,pp.
  536-539